Journal of the American Academy of Audiology最新文献

Background: Dominant deafness-onychodystrophy (DDOD) syndrome is a rare genetic disorder characterized by sensorineural hearing loss and the absence or hypoplasia of nails, associated with defects in the ATP6V1B2 gene. This gene defect significantly affects hearing function, leading to congenital severe-to-profound hearing loss. Purpose: We present the comprehensive cochlear implant (CI) outcome of a 5-year-old child with DDOD syndrome who received bilateral sequential CIs at the ages of 1  and 4 years. Research Design: Case study. Data Collection and Analysis: Retrospective chart review of aural-communication and language performance. Results: After the follow-up following the first CI, there was improvement in auditory, language, and cognitive abilities. At 41 months after the first CI, the child received the second CI. Although his language ability still lagged behind, his auditory and communication performance continued to improve after bilateral CI surgery. He obtained 95 percent on the Parents' Evaluation of Aural/Oral Performance of Children scale at the last follow-up. Conclusions: Children with DDOD syndrome (ATP6V1B2 c.1516C > T) receiving bilateral CIs can enhance aural and communication skills.

Purpose: The primary aim of this preliminary study was to explore the relationship between five attention domains, cognitive flexibility, and speech-in-noise (SIN) performance in both auditory-only (AO) and audiovisual (AV) modalities. Methods: Ten younger and 10 middle-aged adult participants who had standard pure-tone averages no greater than 15 dB HL completed the following three behavioral measures. The Multimodal Lexical Sentence Test for Adults (Kirk et al, 2012) was used to evaluate speech-in-noise performance in AO and AV modalities. Two lists of 12 sentences were presented at a fixed 0-dB signal-to-noise ratio for each of the AO and AV conditions. The Attention Processing Training test (Sohlberg and Mateer, 2005) was administered to each participant, presented bilaterally at 60 dB HL via insert earphones to assess five domains of attention: sustained attention (I), complex sustained attention (II), selective attention (III), divided attention (IV), and alternating attention (V). The Comprehensive Trail-Making Test, Second Edition (Reynolds, 2019) was administered to assess participants' inhibitory control and cognitive flexibility, which are heavily influenced by attention. Results: Correlation and regression analyses of these sample data indicated a significant link between alternating attention and SIN performance in the auditory modality in the younger adults. This link was not observed in middle-aged adults, nor for audiovisual SIN performance. Conclusions: In this study sample, younger individuals with better alternating attention abilities were able to better use contextual information to understand speech in noisy situations without visual context support. The younger adults capitalized on their alternating attention capacities to improve their auditory-only SIN performance, whereas the middle-aged adults did not demonstrate this ability despite similar (sometimes better) alternating attention scores. Alternating attention was not used in the AV modality in either group, possibly due to the simultaneous demand of visual and auditory inputs.

Background: Pulsatile tinnitus (PT) is a form of tinnitus described as a rhythmic beating or whooshing in the ear or ears. Causes of PT can be quite variable and difficult to assess. Purpose: Ambient pressure tympanography (APT) can aid in diagnosing the pathology of the temporal bone that may cause PT. APT measures compliance of the tympanic membrane (TM) over the span of 15-60 s with ambient pressure in the ear canal. The reflex decay test protocol includes APT to measure changes in admittance with a tonal stimulus. Using a contralateral reflex decay test setup without the use of a contralateral stimulus (outside of the ear at 75 dB), any movement of the tympanic membrane may be considered internally produced. Research Design: When a patient experiences tinnitus that is nonpulsatile, contralateral reflex decay testing without the stimulus tone should reveal a flat-line recording because there is no change in pressure or volume of the middle ear space caused by the stimulus. When a patient experiences PT, the TM may appear to be moving rhythmically secondary to pressure and volume changes in the middle ear space. Study Sample: Three adult patients (age older than 18 years) who recently presented to an audiology and otolaryngology clinic and reported PT symptoms were evaluated using APT. Intervention: Interventions were not studied during this case series. Data Collection and Analysis: GSI TympStar Pro Equipment measured TM movement over 15-s intervals. Contralateral reflex decay test settings were utilized with the contralateral stimulus reduced to 75 dB and kept outside of the patient's ear canal to avoid stimulating the system. The measuring probe tip was inserted in the symptomatic ear canal to maintain pressure and observe any internal movement in the middle ear. Results: APT of our patient cohort revealed fluctuating changes in the middle ear cavity volume with a rhythmic pattern that corresponded with the heartbeat. Imaging studies that were performed for each patient demonstrated the likely cause of PT. Conclusion: APT is an efficient clinical tool used to evaluate PT. If a rhythmic change in admittance that corresponds with the patient's heart rate is noted, then further imaging of the temporal bone to determine possible causes may be warranted.

Background: Labyrinthitis, an inner ear infection affecting the membranous labyrinth, typically presents with symptoms such as dizziness, nausea, vomiting, tinnitus, and hearing loss. However, there is limited epidemiological data on this condition. Purpose: This case report presents the results of audiovestibular evaluation and vestibular rehabilitation regarding unilateral sudden hearing loss and acute vestibular syndrome caused by rare labyrinthitis in a pediatric patient. Research Design: This is a case report. Study Sample: The study sample included a 5-year-old girl with unilateral sudden hearing loss and acute vestibular syndrome. Results: The patient with labyrinthitis, diagnosed early after comprehensive audiological and vestibular evaluation, benefited from medical treatment and vestibular rehabilitation. Conclusions: This report objectively focuses on the importance of early medical treatment, regular audiovestibular follow-up, and vestibular rehabilitation in a pediatric patient with labyrinthitis.

Objective: CHARGE syndrome (CS) is a genetic disorder caused by pathogenic variants within chromodomain helicase DNA-binding protein 7 (CHD7). The classical presentation includes coloboma, congenital heart defects, atresia of the choanae, retardation of development, genital hypoplasia, and ear anomalies. Clinical presentation varies widely by type and severity. Structural anomalies of the ear cause hearing loss in 93 percent of individuals with CS. Factors to predict the type and degree of hearing loss among individual patients with CS have not been identified. Identifying factors would ensure patients receive early intervention and allow health care providers to accurately counsel on expectations. Research Design: To identify factors correlated with certain types and degrees of hearing loss, a retrospective chart review of 57 pediatric individuals with CHD7 disease-causing variants from the CHARGE Center at Cincinnati Children's Hospital Medical Center was conducted. All data were manually extracted from participants' medical records, as well as the CHARGE Clinic REDcap database. Type and degree of hearing loss were compared to the type of CHD7 variant and craniofacial anomalies. Results: Within our cohort, 97 percent of individuals experienced hearing loss (HL), with sensorineural (SNHL) being the most common type. Eighty-three percent experienced severe-to-profound HL in at least one ear, a higher prevalence than previously reported. Temporal bone scan results were available for 34 participants with HL, and structural anomalies were seen in 79 percent of individuals. Type of HL did correlate with CHD7 variant type (n = 100, p = 0.002). The most striking relationship is an increased risk of SNHL with CHD7 haploinsufficiency due to nonsense or frameshift variants. Conclusions: Regardless of the type or location of CHD7 variant, patients with CS are at risk for HL and should undergo temporal bone analysis as part of their initial workup. Such findings continue to expand providers' understanding of CS and will improve the management of patients.

Background: The AzBio Sentence-in-Noise Test was developed in 2011 and was successful in minimizing speech-recognition ceiling effects, giving clinicians and researchers a more accurate representation of a listener's speech-in-noise recognition. Recently, the Spanish version of the AzBio corpus was developed as a sentence-recognition test that could similarly be used to reduce speech-recognition ceiling effects in Spanish-speaking patients. The developers of the AzBio in Spanish included researchers and clinicians from the United States and Colombia. Purpose: The aim of this study was to determine whether the AzBio test batteries in English and Spanish are comparable in difficulty to proficient Spanish-English bilingual adults residing in the United States. Research Design: The study was designed as a standard group comparison. Study Sample: Participants included 20 Spanish-English bilinguals between the ages of 18 and 30 years with hearing thresholds no greater than 25 dB HL in both ears. Data Collection and Analysis: Participants listened to three lists of 20 sentences from the AzBio Sentence-in-Noise Tests in English and in Spanish over two test sessions. Sentences were presented at a +5 dB signal-to-noise ratio in 10-talker babble. Sentence-recognition scores were calculated from total words repeated correctly out of total words presented for all three lists (60 sentences in total) in each language condition. A language experience survey was used to quantify and explore language experience in different dialects of Spanish. Results: Our results indicate that bilingual listeners scored similarly on the English and Spanish test corpora on the group level. On an individual level, participants who spoke a Colombian Spanish dialect were among the highest-performing listeners for the Spanish test corpus and among the lowest-performing listeners for the English corpus. Conclusions: The AzBio in Spanish is a highly valuable clinical tool for evaluating speech recognition in Spanish-speaking patients. Our results suggest that listeners who spoke a Colombian Spanish dialect, consistent with the location where the AzBio in Spanish test was developed, tend to perform better on the Spanish version of the test compared to the English version of the test. Thus, dialectical factors may affect sentence-recognition scores on the AzBio in Spanish corpus. Clinicians in the United States must consider dialect when administering this test corpus because the most common dialect in the United States is Mexican Spanish. Future research should evaluate the education level of listeners to determine the impact of language-specific vocabulary on sentence-recognition performance on both AzBio language corpora.